Abstract
The persistence of abnormal coagulation test results and bleeding after standard treatment in the setting of disseminated intravascular coagulation (DIC) can pose a significant challenge. Recombinant Factor VIIa (Novo Nordisk) has been used in the pediatric population for treatment of hemophilia patients with inhibitors and in various other instances as a single agent to manage coagulopathy. (1) The use of NovoSeven and FEIBA (Baxter Hyland Immune) has been reported in use of adult patients with factor deficiencies and coagulation factor inhibitors. (2) The synergistic use of NovoSeven and FEIBA to improve clinical bleeding caused by DIC in a pediatric patient is being described.
A 15 year old African American male presented with a 2 month history of leg pain and a WBC of 400K. He was diagnosed with Philadelphia chromosome positive CML and treated with Allopurinol and Imatinib. After 7 months on Imatinib, he presented in septic shock. His WBC increased to 50.8K with 44% myeloblasts. Flow cytometry confirmed myeloid blast crisis. The patient was admitted to the PICU on broad spectrum antibiotics. Within 12 hours of admission, he required ventilatory assistance. Central lines were placed in the right subclavian vein, right femoral vein and left radial artery. He began bleeding from his nostrils, endotracheal tube and all of his central line sites. Mitoxantrone and Cytarabine were started. Patient was noted to have DIC with PT> 20 sec, APTT> 40 sec, platelets of 23K and D-Dimers of 11.86 (normal <0.42 mcg/ml). His Fibrinogen level was maintained above 300mg/dl during the first 8 days of admission. By hospital day 2, the patient received numerous units of FFP, platelets and PRBC, but he continued to bleed. NovoSeven was started due to volume concerns and increased bleeding. He had improvement in his coagulation tests but no improvement in his bleeding. On hospital day 3, NovoSeven was given intravenously alternating with FEIBA. The patient received NovoSeven 100 micrograms/kg IV q 12 hours. About 6 hours from the NovoSeven dose, FEIBA was given on a q 12 hour schedule at 50 units/kg IV. Significant improvement was seen with the coagulation profile immediately and the bleeding improved in 6 hours. The patient was discharged from the PICU 15 days from his admission with no clinical adverse events associated with the administration of the NovoSeven and FEIBA. He has subsequently tolerated a haploidentical transplant from his sister and he remains in continuous remission.
Nigel Key and colleagues have described improved clinical clotting response with prothrombin complex concentrate (PCC) and NovoSeven. They hypothesized that the coagulation proteins in PCC have a longer half life impacting on the effectiveness of NovoSeven. (2) The use of FEIBA and NovoSeven is risky without a widely available clinical assay to assess responsiveness to NovoSeven. This anecdotal report suggests that FEIBA and NovoSeven can be given sequentially without adverse thrombotic events. Further study in monitoring NovoSeven and using sequential agents may be helpful in patients who prove unresponsive to NovoSeven alone.
Control of disseminated intravascular coagulation in Klippel-Trenaunay-Weber syndrome using enoxaparin and recombinant activated factor VIIa: a case report
